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Hemolysis-Associated Pulmonary Hypertension in Thalassemia
aDepartment of Emergency Medicine, Children's Hospital & Research Center at Oakland, Oakland, California 94609, USA bChildren's Hospital & Research Center at Oakland, Oakland, California 94609, USA cCritical Care Medicine Department, Clinical Center, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20824, USA dVascular Therapeutics Section, Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20824, USA ePediatric Clinical Research Center, Children's Hospital & Research Center at Oakland, Oakland, California 94609, USA fDepartment of Hematology-Oncology, Children's Hospital & Research Center at Oakland, Oakland, California 94609, USA
Address for correspondence: Claudia R. Morris, M.D., Department of Emergency Medicine, Children's Hospital & Research Center at Oakland, 747 52nd Street, Oakland, California 94609. Voice: 510-428-3259; fax: 510-450-5836. claudiamorris{at}comcast.net
Accumulating evidence supports the existence of a condition involving hemolysis-associated pulmonary hypertension (PHT). Hemolysis-induced release of cell-free hemoglobin and red blood cell arginase, resulting in impaired nitric oxide bioavailability, endothelial dysfunction, and PHT, has been reported in sickle cell disease. Since thalassemia is also a condition of chronic hemolysis, these patients are at risk. The data demonstrate that hemolysis-induced dysregulation of arginine metabolism and PHT also occurs in thalassemia. Erythrocyte release of arginase during hemolysis contributes to the development of PHT. Therapies that maximize arginine and nitric oxide bioavailability may benefit patients with thalassemia.
Key Words: thalassemia • nitric oxide • hemolysis • sickle cell disease This article has been cited by other articles:
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